=CVID. Another terribly named condition.

Usually presents in adulthood but about 20% in childhood. Typically recurrent infections of ears, sinuses, lungs – usual bugs, not funny ones.

Bronchiectasis may develop. In some cases granulomas develop.

Lymphadenopathy +/- splenomegaly is sometimes a feature.

Autoimmunity is an important feature – low red cells or platelets, thyroid disease.

Enteropathy and arthritis can be seen.

Diagnosis

Low IgG, usually IgA, sometimes IgM. Functional antibodies (to pneumococcus, tetanus, Hib) low.

Treatment

Immunoglobulin replacement – IVIG or subcut immunoglobulin, regularly.

Prophylactic antibiotics in some cases. Screen for infections esp chronic GI.